Reactivations in Multisystem Langerhans Cell Histiocytosis: Data of the International LCH Registry - 07/03/14
, Manuel Steiner, MD b, Ulrike Pötschger, PhD b, Maurizio Aricò, MD c, Jorge Braier, MD d, Jean Donadieu, MD e, Nicole Grois, MD b, Jan-Inge Henter, MD, PhD f, Gritta Janka, MD g, Kenneth McClain, MD, PhD h, Sheila Weitzman, MD i, Kevin Windebank, MD j, Stephan Ladisch, MD k, Helmut Gadner, MD, FRCP (Glasgow) a, bInternational LCH Study Group
Reprint requests: Milen Minkov, MD, PhD, St. Anna Children's Hospital, Kinderspitalgasse 6, A-1090Vienna,AustriaRésumé |
Objective |
To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality.
Study design |
Retrospective analysis of 335 patients with MS-LCH and documented complete disease resolution (NAD1).
Results |
The probability of a reactivation within 5 years of NAD1 was 46%. The first reactivation occurred within 2 years after NAD1 in most of the patients. Of 134 events, 35% were confined to skeleton, 24% were single-system nonbony lesions, 24% were multisystem reactivations without risk-organ involvement, and 10% with risk-organ involvement. In 7%, the location was unspecified. Only 3 deaths (2.2%) were documented within the context of a first reactivation. Second disease resolution (NAD2) was achieved in 85% of the cases. The probability of a second reactivation within 5 years of NAD2 was 44%. The risk for permanent consequences in patients with reactivations was higher, compared with patients without reactivation (RHR 2.2, P = .046).
Conclusions |
Reactivation is a frequent and early event in MS-LCH, but involvement of risk organs at reactivation is rare and mortality is minimal. However, reactivations increase the risk for permanent consequences by about 2-fold. Prospective trials targeting reduction of acute morbidity and permanent disabilities through nontoxic treatment of the reactivations are warranted.
Le texte complet de cet article est disponible en PDF.Abbreviations : DI, LCH, SS-LCH, MS-LCH, RO+, RO−, NAD1, NAD2, PC, R1, R2, SS-R, MS-R
Plan
| The authors declare no real or perceived conflicts of interest. |
Vol 153 - N° 5
P. 700 - novembre 2008 Retour au numéro
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